Multifocal Neurological Signs Research Articles

Insights into the clinical presentation, diagnostics and outcome in dogs presenting with neurological signs secondary to infection with Neospora caninum: 41 cases (2014-2023).

To describe the clinical signs and outcome of a large cohort of dogs presenting with neurological signs secondary to Neospora caninum infection. Retrospective review of cases presenting to two UK referral centres with neurological signs secondary to N. caninum infection between 2014 and 2023. Presenting signs, diagnostic test results, treatment, short- and long-term outcome analysed. A total of 1690 cases were assessed for eligibility. Forty-four cases with a diagnosis of neosporosis were obtained. Three cases were then excluded due to non-neurological presentations (two hepatitis and one myocarditis). A total of 41 cases were included in the study. Cerebello-vestibular signs predominated; however, presenting clinical signs were varied and the neurolocalisation was often multifocal in nature (46.3%), making neosporosis an important differential diagnosis for meningoencephalitis of unknown origin. Complete clinical improvement was rare (5.6%), and relapses were common (27.8% cases with follow-up). Neosporosis remains an important differential diagnosis for dogs at any age presenting with multifocal neurological signs. The outcome is considered poor and relapse rate is high.

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease

Patient: Male, 17-month-old Final Diagnosis: Fluid attenuated inversion recovery (FLAIR) imaging showing cortical hyperintense lesions in myelin-oligodendrocyte-glycoprotein (MOG) associated encephalitis with seizures (FLAMES Symptoms: Encephalopathy • fever • left sided weakness • seizures Medication: — Clinical Procedure: Plasmapharesis Specialty: Neurology Objective: Rare disease Background:The relatively new autoimmune disorder, anti-myelin oligodendrocyte glycoprotein (MOG) disease is particularly interesting because of its broad range of presentations. This entity’s appearance on magnetic resonance imaging (MRI) of the brain often makes identifying this disease a challenging process. Younger patients tend to present with an acute disseminated encephalomyelitis picture, with encephalopathy and multifocal neurological signs, while older patients are more likely to present with optic neuritis. We, however, report an atypical case of a patient who presented with encephalopathy, seizures, and significant cortical and subcortical gray matter involvement and was found to have anti-MOG positivity in serum.Case Report:A 17-month-old previously healthy boy presented to Emergency Department with fever, lethargy, vomiting, and left-sided weakness. Eventually, he required intubation due to a prolonged seizure. Continuous electroencephalogram captured several focal seizures, and MRI of the brain showed cortical and subcortical T2 hyperintensities. After extensive laboratory evaluation, he tested positive for anti-MOG antibody. He was empirically started on high-dose intravenous pulse methylprednisolone, followed by plasma exchange, given the poor response to the intravenous steroids. At the 5-month follow-up, the results of the neurological examination had dramatically improved, and MRI findings had largely resolved.Conclusions:This case highlights the importance of suspecting anti-MOG antibody-mediated encephalitis, even while ruling out infectious etiologies, in children presenting with encephalopathy, seizures and MRI abnormalities. Prompt recognition would allow for less delay in treatment and hopefully improve prognosis.

Immediate and Short-term Outcome of Acute Disseminated Encephalomyelitis (ADEM) after corticosteroid therapy

Background : Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system (CNS) occurring predominantly but not exclusively in children. The presence of severe disturbance of consciousness together with multifocal neurological symptoms and signs especially in a child under 10 years of age; favours a diagnosis of ADEM. Neuroimaging is essential investigation for establishing the diagnosis of ADEM. High-dose steroid treatment frequently results in a dramatic clinical improvement.
 Objective: This study aimed to describe our experience with Acute Disseminated Encephalomyelitis (ADEM) focusing on the clinical features, neuroimaging, and outcome after treatment with high-dose steroid.
 Method: This retrospective observational study was conducted in children who were admitted as inpatients in Paediatric Neurosciences Department of Dhaka Shishu Hospital (DSH) with the diagnosis of ADEM during the period of 2017- 2019. All patients got high dose IV methylprednisolone for 5 days followed by 2 weeks tapering dose of steroid. Outcome was observed on the 7th day of treatment and 6 months after discharge from hospital.
 Result: A total of 16 patients who were diagnosed as ADEM were included in this study. Twelve children (75%) had a recent upper respiratory tract illness. Patients presented most often with motor deficits (88%) and secondly with altered consciousness (63% ) followed by seizure ( 50% ). MRI brain was the preferable neuroimaging which mostly (41.7%) showed multifocal symmetrical demyelination patch. On the 7th day of steroid treatment, significant improvement in motor function and level of consciousness occurred (p values were 0.001% and 0.014% respectively). After 6 months of discharge from hospital; no motor deficit, age-appropriate cognitive level and no seizure in 71%, 57% and 71% respectively.
 Conclusion: High dose corticosteroid treatment in ADEM is significantly effective in improvement of motor impairment and consciousness level on immediate follow up.
 Northern International Medical College Journal Vol.11 (2) Jan 2020: 468-470

Clinical, MRI, and Histopathological Features of Hypothalamic Neuronal Hamartoma in a Young Vizsla.

Human hypothalamic neuronal hamartomas are rare, nonprogressive, congenital malformations of the hypothalamus that do not expand or metastasize to other locations. A 1 yr old female vizsla was presented for progressive intracranial multifocal neurological signs present since adoption at 3 mo of age. MRI of the brain showed an ill-defined, intra-axial, space-occupying, nonenhancing lesion located in the ventral middle cranial fossa. Histopathological examination was consistent with hypothalamic neuronal hamartoma. This is the first report describing clinical, imaging, and histopathological features of a hypothalamic neuronal hamartoma in a dog. These findings are compared with their human counterparts.

3‐T MRI of protothecosis encephalic lesions in a Scottish shepherd dog

A four‐year‐old Scottish Shepherd dog was referred for acute onset of disorientation, blindness and chronic diarrhoea. The neurological examination was consistent with a multifocal neurolocalisation involving the prosencephalon and the brainstem. The ophthalmological exam showed bilateral chorioretinitis with retinal detachment. The brain MRI showed periventricular and left caudate nucleus lesions, increased thickness of the left III, IV, VI and opthalmic branch of V cranial nerves and bilateral multifocal lesions in the temporal and masseter muscles. Cerebrospinal fluid examination showed mild mixed pleocytosis. Faecal and urine cultures were positive for Prototheca zopfii genotype 2. Despite starting itraconazole therapy, the dog's conditions deteriorated and the owner elected euthanasia. Protothecosis should be suspected in young dogs with multifocal neurological signs, blindness and chronic diarrhoea.MRI study of the central nervous system (CNS), together with blood exam, cytology and cultures, is a useful tool to evaluate the CNS in disseminated form of Prototheca infection.

Distinct mutation in the feline coronavirus spike protein cleavage activation site in a cat with feline infectious peritonitis-associated meningoencephalomyelitis.

Case summaryThis report describes a cat with chronic, progressive, non-painful, non-lateralizing multifocal neurologic clinical signs associated with feline infectious peritonitis (FIP). The cat initially presented as underweight, despite a good appetite, and a complete blood count showed non-regenerative anemia. Three months later the cat was returned having developed ataxia and paraparesis, which then progressed over 2 months to tetraparesis, tail plegia, urinary and fecal incontinence, and titubation. Histologic examination of the tissues with subsequent immunohistochemistry confirmed FIP-associated meningoencephalomyelitis following necropsy. Molecular analysis of the coronavirus spike protein within the tissues identified a specific, functionally relevant amino acid change (R793M), which was only identified in tissues associated with the central nervous system (ie, brain and spinal cord).Relevance and novel informationThis case report describes an early presentation of a cat with primarily neurologic FIP, with molecular characterization of the virus within various tissues.

Intravascular large B-cell lymphoma mimicking central nervous system vasculitis

Intravascular large B-cell lymphoma (IVBCL) represents a rare subtype of extranodal diffuse large B-cell lymphoma characterized by selective growth of neoplastic cells within the lumen of small and medium-sized vessels. The clinical spectrum is heterogeneous dominated by neurological and skin manifestations. Intra-vitam diagnosis still remains challenging and usually requires brain biopsy since no pathognomonic neuroradiological findings do exist for IVBCL.We report on the case of a 65-year old male patient presenting with multifocal neurological signs and symptoms including cognitive deficits, aphasia and paraparesis as leading features. Imaging findings were suggestive for cerebral vasculitis prompting initiation of steroid treatment. After initial stabilization the patient deteriorated in spite of systemic steroid treatment, developed severe sepsis and finally died from multi-organ failure. Histopathological findings revealed CD20 positive lymphoma cells in small- and middle-sized vessels of the cerebrum, the lungs and the skin diagnostic for IVBCL.This case should raise the alertness for consideration of IVBCL as a differential diagnosis resulting in early brain biopsy, if no etiology for CNS vasculitis is evident.

Intravascular Large B-Cell Lymphoma Mimicking Central Nervous System Vasculitis

Intravascular large B-cell lymphoma (IVBCL) represents a rare subtype of extranodal diffuse large B-cell lymphoma characterized by selective growth of neoplastic cells within the lumen of small and medium-sized vessels. The clinical spectrum is heterogeneous dominated by neurological and skin manifestations. Intra-vitam diagnosis still remains challenging and usually requires brain biopsy since no pathognomonic neuroradiological findings do exist for IVBCL. We report on the case of a 65-year old male patient presenting with multifocal neurological signs and symptoms including cognitive deficits, aphasia and paraparesis as leading features. Imaging findings were suggestive for cerebral vasculitis prompting initiation of steroid treatment. After initial stabilization the patient deteriorated in spite of systemic steroid treatment, developed severe sepsis and finally died from multi-organ failure. Histopathological findings revealed CD20 positive lymphoma cells in small- and middle-sized vessels of the cerebrum, the lungs and the skin diagnostic for IVBCL. This case should raise the alertness for consideration of IVBCL as a differential diagnosis resulting in early brain biopsy, if no etiology for CNS vasculitis is evident.

Adult autologous mesenchymal stem cells for the treatment of suspected non-infectious inflammatory diseases of the canine central nervous system: safety, feasibility and preliminary clinical findings.

BackgroundNon-infectious inflammatory diseases of the canine central nervous system (CNS) are common idiopathic disorders grouped under the term meningoencephalomyelitis of unknown origin (MUO). Ante mortem diagnosis is achieved via assessment of clinical signs, magnetic resonance imaging (MRI), and cerebrospinal fluid (CSF) analysis, but the definitive diagnosis needs histopathological examination. MUO are mostly considered as autoimmune CNS disorders, so that suppressing the immune reaction is the best management method for patients. Mesenchymal stem cells (MSCs) are under investigation to treat autoimmune and degenerative disorders due to their immunomodulatory and regenerative properties. This study aims to verify the safety, feasibility, and efficacy of MSCs treatment in canine idiopathic autoimmune inflammatory disorders of the CNS.MethodsEight dogs presented with acute onset and rapid progression of multifocal neurological signs were selected to the study. In all patients’ physical and neurological examinations, MRI and CSF analyses were performed. Clinical diagnosis in all cases was MUO. All selected dogs responded initially to immunosuppressive drugs (prednisone and a combination of prednisolone and cytosine arabinoside) but developed undesirable side effects. For all eight dogs, the owners considered euthanasia but accepted cell therapy as a last possibility. Autologous bone marrow MSCs (BMMSCs), isolated, cultured, and expanded, were administered by intrathecal (IT) injection in the cisterna magna intravenously (IV) and by intra-arterial (IA) injection in the right carotid artery. Adverse effects and clinical response were monitored for 6 months up to 2-year follow-up.ResultsThe use of autologous BMMSCs in dogs with MUO was safe for IT, IV, and IA injections. No major short- or long-term adverse effects were registered. All the dogs presented early improvement in their general and neurological conditions, with particular effect on cervical pain. The group of dogs treated by IT+IA administration showed a shorter time of reaction to therapy compared to the group treated by IT+IV administration.ConclusionsMSCs treatment in dogs affected by MOU is safe and feasible. A larger group of dogs is needed to confirm these results as well as CNS histology in order to better understand the underlying mechanisms.

Leptomeningeal Metastases in Solid Tumors

BACKGROUND: Leptomeningeal metastases are diagnosed in 1% to 8% of patients with cancer. The most common histology is leukaemia, lymphoma, breast carcinoma, lung carcinoma and melanoma. The aim of this study is to show the appearance of leptomeningeal metastases in lung and breast cancer with specific biological features.AIM: The study is a review of two cases - breast and lung carcinoma with specific biological features who eventually have developed leptomeningeal metastases.CASE PRESENTATION: Although CNS metastasis most commonly occurs in those with known systemic metastases, our patient had biological indolent disease (nor hormone negative or HER 2 positive and no metastatic disease) and still LM did occur. LM remains a devastating complication of cancer with classic presentation with multifocal neurologic signs and symptoms (like the case with lung cancer), but many patients also have evidence of impaired CSF flow, and hydrocephalus may be the only presenting manifestation of LM.CONCLUSION: The prognostic factors associated with survival in patients with LM metastasis are not certain as well as right treatment for these patients which is still a big challenge. Due to the lack of randomized studies and especially of studies referring to one specific primary tumor, there is currently no generally accepted standard of care in the treatment of LM metastases.

Leptomeningeal Metastases in Solid Tumors

BACKGROUND: Leptomeningeal metastases are diagnosed in 1% to 8% of patients with cancer. The most common histology is leukaemia, lymphoma, breast carcinoma, lung carcinoma and melanoma. The aim of this study is to show the appearance of leptomeningeal metastases in lung and breast cancer with specific biological features.AIM: The study is a review of two cases - breast and lung carcinoma with specific biological features who eventually have developed leptomeningeal metastases.CASE PRESENTATION: Although CNS metastasis most commonly occurs in those with known systemic metastases, our patient had biological indolent disease (nor hormone negative or HER 2 positive and no metastatic disease) and still LM did occur. LM remains a devastating complication of cancer with classic presentation with multifocal neurologic signs and symptoms (like the case with lung cancer), but many patients also have evidence of impaired CSF flow, and hydrocephalus may be the only presenting manifestation of LM.CONCLUSION: The prognostic factors associated with survival in patients with LM metastasis are not certain as well as right treatment for these patients which is still a big challenge. Due to the lack of randomized studies and especially of studies referring to one specific primary tumor, there is currently no generally accepted standard of care in the treatment of LM metastases.

Quantitative evaluation of the severity of acute illness in adult patients with tick-borne encephalitis.

The aim of the present study was to quantify the severity of acute illness in patients with tick-borne encephalitis and to ascertain this approach by comparing it to standard clinical assessment. We designed scoring system for quantification of the severity of acute illness in patients with tick-borne encephalitis. Certain number of points was allotted to the presence, intensity, and duration of individual symptoms/signs. According to the obtained score the disease was classified as mild, moderate, and severe. Tick-borne encephalitis was assessed clinically as mild when only signs/symptoms of meningeal involvement were found, moderate in case of monofocal neurological signs and/or mild to moderate signs/symptoms of central nervous system dysfunction, and severe in patients with multifocal neurological signs and/or symptoms of severe dysfunction of central nervous system. By designed scoring system 282 adult patients, 146 males and 136 females, average aged 52.2 ± 15.5 years (range 15–82 years), with confirmed tick-borne encephalitis, were prospectively assessed. In 279/282 (98.9%) patients the severity according to clinical assessment matched with the score ranges for mild, moderate, and severe disease. The proposed approach enables precise and straightforward appraisal of the severity of acute illness and could be useful for comparison of findings within/between study groups.

Acute hemorrhagic encephalopathy - diagnostic challenges in a pediatric case

Background Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare form of Acute disseminated encephalomyelitis (ADEM), due to acute cerebral vasculitis. The symptomatology consists in similar neurological findings, (meningismus, headache, seizures, multifocal neurologic signs, asymmetrical neurological deficits and coma) with rapid onset of encephalopathy and biphasic evolution. Although previous respiratory disease was registered days before this condition, establishing etiology is quite a challenge, viruses being incriminated.

Encephalopathic Susac’s Syndrome associated with livedo racemosa in a young woman before the completion of family planning

BackgroundSusac’s Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO) and hearing loss (HL). Histopathologically, SS is characterised by a microangiopathy, and some observations suggest that an immune-mediated damage of endothelial cells might play a role. These findings also implicate a similarity between SS and other autoimmune diseases, most notably juvenile dermatomyositis (JDM). However, SS and JDM are commonly thought to affect distinct and non-overlapping sets of organs, and it is currently not clear how these specificities arise. Moreover, in the absence of clinical trials, some authors suggest that therapeutic approaches in SS should rely on the model of other autoimmune diseases such as JDM.Case presentationHere, we report a case of SS in a 32-year-old pregnant woman. She initially was admitted to the hospital with subacute severe encephalopathy and multifocal neurologic signs. As cranial magnetic resonance imaging (MRI) revealed multifocal white matter lesions including the corpus callosum, erroneously a diagnosis of multiple sclerosis (MS) was made, and intravenous methylprednisolone (IVMP) therapy was initiated. A few days later, an exanthema appeared on the trunk and extremities, which was diagnosed as livedo racemosa (LR). Several weeks later, the patient was readmitted to the clinic with an obscuration of her left visual hemifield and a bilateral HL. Ophthalmologic examination revealed extensive ischemic damage to both retinae. Now the correct diagnosis of SS was established, based on the above triad of clinical symptoms in conjunction with typical MRI and fundoscopic findings. When SS was diagnosed, the standard therapy with intravenous cyclophosphamide (IVCTX) was not instituted because of a significant risk of permanent infertility. Instead, sustained control of disease activity could be achieved with a therapeutic regime combining prednisolone, intravenous immunoglobulins (IVIG), mycophenylate mofetil (MM), and methotrexate (MTX).ConclusionsAn association with LR has only been described in very few cases of SS before and further underlines the pathogenetic relationship between SS and other autoimmune diseases such as JDM. In young women with SS and the desire for a child the combination of MM and MTX may represent a reasonable alternative to IVCTX.

IMAGING DIAGNOSIS—CONVENTIONAL AND FUNCTIONAL MAGNETIC RESONANCE IMAGING OF A BRAIN ABSCESS IN A GOAT

A 2-month-old female goat was presented for depressed mental status and multifocal central neurologic signs 3 weeks after hot-iron disbudding. Conventional magnetic resonance imaging (MRI) findings included a large intra axial mass in the left frontal lobe that was T2 hyperintense and T1 hypointense centrally with a contrast-enhancing peripheral capsule and perilesional T2 hyperintensity. A restrictive pattern was present in diffusion-weighted imaging. Magnetic resonance spectroscopy demonstrated an increased amount of succinate, acetate, amino acids, lipids; minimal amounts of lactate; and decreased amounts of N-acetyl aspartate and choline. A cerebral abscess due to Trueperella pyogenes was confirmed from necropsy and tissue culture.

Leptomeningeal Metastasis From Systemic Cancer

Leptomeningeal metastasis (LM) is an uncommon, usually late, complication of cancer. This article discusses the clinical presentation, diagnosis, prognosis, and treatment of LM. Neither gadolinium-enhanced MRI nor CSF cytology is adequately sensitive to diagnose all cases of LM. Patients with LM classically present with multifocal neurologic symptoms and signs, and the diagnosis is made via MRI, CSF cytologic analysis, or both. Treatment is palliative in nature and can involve focal radiation to symptomatic sites, systemic chemotherapy with agent(s) that cross the blood-brain barrier, or intrathecal delivery of chemotherapy. Patients may present with or later develop signs of hydrocephalus; ventriculoperitoneal shunting can provide symptomatic relief in certain patients. Overall prognosis is poor, with a median survival typically in the range of 2 to 3 months.

Meningeal carcinomatosis in solid tumors

The involvement of the leptomeninges by metastatic tumors can be observed in solid tumors, in which case it is termed meningeal carcinomatosis (MC), and in lymphoproliferative malignant disease. It is more common in breast and lung cancer, as well as melanoma, with adenocarcinoma being the most frequent histological type. MC is usually a late event, with disseminated and progressive disease already present and, it is characterized by multifocal neurological signs and symptoms. Diagnosis is based on the evaluation of clinical presentation, cerebrospinal fluid and neuroimaging studies. The better systemic disease control is observed with new therapeutic agents, and the development of neuroimaging methods is responsible for the increasing incidence of such metastatic evolution. Intrathecal chemotherapy is generally the treatment of choice, although frequently palliative. Prognosis is guarded, although a higher performance status may indicate a subgroup of patients with a more favorable outcome.

Tek Hemisferi Tutan Akut Dissemine Ensefalomiyelit: Olgu Sunumu

Acute disseminated encephalomyelitis (ADEM) is a rare idiopathic inflammatory demyelinating disease of the central nervous system (CNS), which usually occurs following a viral or bacterial infection, or vaccination. Characteristically, ADEM presents with acute encephalopathy with accompanying multifocal neurological symptoms and signs. Due to unavailability of a specific biological marker, neuroimaging is very important in establishing the diagnosis of ADEM. The typical magnetic resonance imaging findings of ADEM are large, multiple and almost symmetrical white matter lesions. Herein, we report a 31-year-old woman presenting with headache, confusion, left hemiparesis and hemihypoesthesia preceded by an infection. The patient fully recovered after intravenous methylprednisolone treatment. MRI revealed white-matter lesions that were typical for ADEM, but the unihemispheric involvment broadened the spectrum of disseminated encephalomyelitis. (Archives of Neuropsychiatry 2010; 47: 166-8)

Intracranial anaplastic astrocytoma in a 19-week-old boxer dog

A 19-week-old, entire female boxer dog was presented for evaluation of chronic, progressive multi-focal neurological signs. Magnetic resonance imaging suggested a large intra-axial mass in the brainstem. Euthanasia was performed and an anaplastic astrocytoma was found at post-mortem examination. This case reports an unusual naturally occurring brain tumour in a young dog.

Intrathecal chemotherapy for treatment of leptomeningeal dissemination of metastatic tumours

Neoplastic meningitis consists of diffuse involvement of the leptomeninges by infiltrating cancer cells, and can be caused by systemic or primary CNS tumours, such as solid cancers or lymphoproliferative malignant disease. Neoplastic meningitis is characterised by multifocal neurological signs and symptoms. Thus, careful neurological examination is needed for diagnosis of secondary diffuse involvement. Survival of patients with neoplastic meningitis is short (3-4 months), although some patients have long-lasting remission. Because most patients with neoplastic meningitis have diffuse systemic disease, treatment is typically palliative. However, more aggressive treatments are available to low-risk patients, which could increase survival. Therefore, identification of low-risk patients is important. Intrathecal chemotherapy is currently the main treatment for patients with neoplastic meningitis, but optimum anticancer chemotherapy is being studied.